• crizanlizumab-tmca (Adakveo^®) intravenous infusion for administration by a healthcare professional

• For reducing the frequency of vasoocclusive crises in adults and pediatric patients 16 years and older with sickle cell disease 

The restricted product(s) may be considered medically necessary when the following criteria are met: 

Initial Criteria for Approval: 

1. The patient is 16 years of age or older; AND
2. The patient has a diagnosis of sickle cell disease (SCD) of any genotype (e.g., HbSS, HbSC, HbS/beta⁰ -thalassemia, HbS/beta⁺ - thalassemia) [medical record documentation required]; AND
3. The patient has had at least 2 vasoocclusive crisis (VOC) events[D] in the last 12 months prior to starting therapy with the requested agent [medical record documentation required]; AND 
4. The requested agent will be used to reduce the frequency of vasoocclusive crises (VOCs) [medical record documentation required]; AND
5. The patient has a baseline hemoglobin (Hgb) level ≥ 4.0 g/dL [medical record documentation required]; AND
6. ONE of the following [medical record documentation required]: 
   1. The patient is currently taking hydroxyurea; OR
   2. The patient has tried and had an inadequate response to hydroxyurea; OR
   3. The patient has an intolerance, FDA labeled contraindication, or hypersensitivity to hydroxyurea; AND
7. The patient will NOT be using crizanlizumab-tmca (Adakveo^® ) in combination with voxelotor (Oxbryta^™) [medical record documentation required]; AND 
8. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., hematologist, SCD specialist) or has consulted with a specialist in the area of the patient’s diagnosis [medical record documentation required]; AND
9. The requested quantity does NOT exceed the maximum units allowed for the duration of approval (see table below); AND
10. For requests for injection or infusion administration of the requested medication in an inpatient or outpatient hospital setting, Site of Care Criteria applies (outlined below)

Duration of Approval: 180 days (6 months)

*VOC events are defined as acute episodes of pain with no cause other than a vasoocclusive event that require a medical facility visit and treatment with oral or parenteral opioids, or parenteral NSAIDs. VOC events may include acute chest syndrome, hepatic sequestration, splenic sequestration, and/or priapism requiring a medical facility visit.

Continuation Criteria for Approval:

1. The patient was approved through Blue Cross NC initial criteria for approval; OR
2. The patient would have met initial criteria for approval at the time they started therapy; AND 
3. The patient has disease improvement while using the requested agent, as demonstrated by a decrease in the frequency of vasoocclusive crisis (VOC) events [medical record documentation required]; AND 
4. The patient will NOT be using crizanlizumab-tmca (Adakveo^® ) in combination with voxelotor (Oxbryta^™) [medical record documentation required]; AND
5. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., hematologist, SCD specialist) or has consulted with a specialist in the area of the patient’s diagnosis [medical record documentation required]; AND 
6. The requested quantity does NOT exceed the maximum units allowed for the duration of approval (see table below); AND 
7. For requests for injection or infusion administration of the requested medication in an inpatient or outpatient hospital setting, Site of Care Criteria applies (outlined below)

Duration of Approval: 365 days (1 year)

FDA Label Reference 

*Maximum units allowed for duration of approval

*Site of Care Medical Necessity Criteria

1. For requests for injection or infusion administration in an inpatient setting, the injection or infusion may be given if the above medical necessity criteria are met AND the inpatient admission is NOT for the sole purpose of administering the injection or infusion; OR
2. For requests for injection or infusion administration in an outpatient hospital setting, the injection or infusion may be given if the above medical necessity criteria are met AND ONE of the following must be met:
   1. History of mild adverse events that have not been successfully managed through mild pre-medication (e.g., diphenhydramine, acetaminophen, steroids, fluids, etc.); OR
   2. Inability to physically and cognitively adhere to the treatment schedule and regimen complexity; OR
   3. New to therapy, defined as initial injection or infusion OR less than 3 months since initial injection or infusion; OR
   4. Re-initiation of therapy, defined as ONE of the following:
      1. First injection or infusion after 6 months of no injections or infusions for drugs with an approved dosing interval less than 6 months duration; OR
      2. First injection or infusion after at least a 1-month gap in therapy outside of the approved dosing interval for drugs requiring every 6 months dosing duration; OR
   5. Requirement of a change in the requested restricted product formulation; AND
3. If the Site of Care Medical Necessity Criteria in #1 or #2 above are not met, the injection or infusion will be administered in a home-based infusion or physician office setting with or without supervision by a certified healthcare professional.

References:

All information referenced is from FDA package insert unless otherwise noted below.

1. Ataga KI, Kutlar A, Kanter J, et al. Crizanlizumab for the prevention of pain crises in sickle cell disease. N Engl J Med. 2017;376:429-39.

Policy Implementation/Update Information

October 2021: Criteria change: Added Site of Care medical necessity criteria. Policy notification given 8/2/2021 for effective date 10/1/2021.

June 2021: Criteria change: Added maximum units; medical policy formatting change. Policy notification given 4/16/2021 for effective date 6/16/2021.

*Further historical criteria changes and updates available upon request from Medical Policy and/or Corporate Pharmacy.